Tuesday, June 10, 2008

Almost everyone I know is confused about exactly what POTS and dysautonomia are. These disorders are extremely difficult to diagnose.Many people (including myself) go many years and dozens of doctors without any diagnosis at all.A lack of diagnosis can be exhausting, disheartening, and frustrating, only increasing the severity of symptoms and disability.It is important that anyone with a so-called “mystery diagnosis” not give up and keep searching for answers until they find someone who can help them.That’s not to say that you will like the diagnosis, but at least you will KNOW and can focus your efforts towards fighting your disease.

This post is crazy long, but aimed at explaining a little more about what these disorders are.It does get a bit “scienc-y”, but I am a vet after all! I will try not to put any other posts as long as this one on in the future unless under extreme duress.

What is Dysautonomia?

Dysautonomia is a dysfunction of the autonomic nervous system (ANS).Some of the disorders that are types of dysautonomia include POTS, neutrally mediated syncope, pure autonomic failure, multiple system atrophy, shy drager syndrome, and familial dysautonomia, among others.

According to the Merck Manual, the autonomic nervous system is the part of the nervous system that supplies the internal organs, including the blood vessels, stomach, intestine, liver, kidneys, bladder, genitals, lungs, pupils and muscles of the eye, heart, and sweat, salivary, and digestive glands. The autonomic nervous system has two main divisions: the sympathetic and the parasympathetic. After the autonomic nervous system receives information about the body and external environment, it responds by stimulating body processes, usually through the sympathetic division, or inhibiting them, usually through the parasympathetic division. The autonomic nervous system controls blood pressure, heart and breathing rates, body temperature, digestion, metabolism (thus affecting body weight), the balance of water and electrolytes (such as sodium and calcium), the production of body fluids (saliva, sweat, and tears), urination, defecation, sexual response, and other processes.

Autonomic disorders commonly cause dizziness or light-headedness due to an excessive decrease in blood pressure when a person stands (orthostatic hypotension). People may sweat Photo courtesy of NIAMS less or not at all and thus become intolerant of heat. The eyes and mouth may be dry. After eating, a person with an autonomic disorder may feel prematurely full or even vomit because the stomach empties very slowly (gastroparesis). Some people pass urine involuntarily (urinary incontinence), often because the bladder is overactive. Other people have difficulty emptying the bladder (urine retention) because the bladder is underactive. Constipation may occur, or control of bowel movements may be lost. The pupils may not dilate and narrow (constrict) as light changes.

What is POTS?

POTS, or Postural Orthostatic Tachycardia Syndrome, is a disorder characterized by a pulse rate that is too fast when the patient stands. POTS is a type of non-familial dysautonomia that is seen most commonly in relatively young (14-45) women (5:1 over men). The cause of each form of POTS is usually unknown. It is thought that approximately 500,000 Americans suffer with from some type of this disorder. It can be completely disabling, keeping many from standing, walking, driving, cooking, concentrating, working, or showering without assistance.While many are lucky enough to be able to manage their symptoms and have a relatively normal life, some people are severely disabled indefinitely.Some of the most common symptoms felt by people with POTS include:

Understanding of POTS pathophysiology remains incomplete. The central finding is upright tachycardia with symptoms of orthostatic intolerance, although hypotension (in children) and resting tachycardia may also be present. That POTS is related to thoracic hypovolemia (low blood volume) is well established. Adults can experience hypertension; some individuals have both high and low BP.

POTS remains a syndrome; only thoracic hypovolemia is needed. Thus, dehydration might serve as a model for POTS. Indeed, a large fraction of patients with POTS have reduced blood volumes and paradoxical disturbances in the renin-angiotensin-aldosterone system.

Conversely, reduced intravascular volume can activate the autonomic nervous system; thus, the hypovolemia may be either the cause or the effect. Similar heart rate, BP, catecholamine (neurotransmitter) changes, and blood volume differences have been described in a subset of patients with POTS but not in others with relative hyporeninism. Supportive evidence from volume loading and vasoconstrictor experiments has shown transient success in remediating POTS. Similar interesting findings have also appeared in the ChronicFatigueSyndrome literature. Although these findings have largely been interpreted within a framework of the hypothalamus-pituitary-adrenal axis, the findings also make sense within the POTS paradigm. (from emedicine Orthostatic Intolerance: An Overview at http://www.emedicine.com/ped/topic2860.htm).

An extremely helpful article for understanding POTS and its different forms and treatments is The Postural Tachycardia Syndrome: A Concise Guide to Diagnosis and Management by Grubb, B.D., Kanjwal,Y, and Kosinski, D.J. from the Journal of Cardiovascular Electrophysiology. 2006;17(1):108-112.According to this article:

The hallmark of these disorders is orthostatic intolerance, defined as the provocation of symptoms on standing, which are relieved when becoming supine. Patients often relate complaints of palpitations, exercise intolerance, fatigue, lightheadedness, tremor, headache, nausea, near syncope, and syncope.[2] Patients may be severely limited as activities such as housework, bathing, and even meals may exacerbate symptoms. Recent studies have shown that many patients with POTS may suffer the same degree of functional impairment as patients with chronic obstructive pulmonary disease or congestive heart failure, yet these patients are often misdiagnosed as having chronic anxiety or panic disorder.[3] POTS is classified as being either primary or secondary. The primary forms are idiopathic and not associated with other diseases. The secondary forms occur in association with a known disease or disorder. Proper recognition of subtypes is essential in management[4] (Fig. 1).

Pharmacotherapy is initiated with the goal of getting patients well enough to pursue reconditioning. No drug is currently approved by the US FDA for the treatment of POTS; therefore, any use of pharmacotherapy is done "off label." Knowing the subtype is important in choosing appropriate pharmacotherapy ( Table 3 ).

In the partial dysautonomic POTS, patient therapy is directed at augmenting fluid volume and increasing vascular resistance. We often begin with fludrocortisone, a mineral corticoid that promotes sodium and fluid retention, and also sensitizes peripheral alpha adrenergic receptors. The drug appears to be most helpful in younger patients. Next, we add a vasoconstrictor such as midodrine, starting at 5 mg orally three times a day.Since patients are most symptomatic early in the morning, we often advise patients to take their midodrine dose approximately 15 minutes prior to getting out of bed. We also advise patients that they can take an extra 5 mg dose as needed if severe breakthrough symptoms occur. If midodrine is not tolerated methylphenidate can be an effective alternative, especially because it comes in several long-acting preparations.

If patients continue to be symptomatic, we add either a serotonin reuptake inhibitor (SSRI) or a norepinephrine reuptake inhibitor. While the SSRIs are very useful in the prevention of neurocardiogenic syncope, the norepinephrine reuptake inhibitors are somewhat more helpful in POTS patients. We usually employ bupropion in the XL form. Of all the currently available SSRIs, we have noted that those with combined serotonin and norepinephrine reuptake inhibition (such as venlafaxine and duloxetine) are often the most effective. A very promising new therapy is pyridostigmine, an acetylcholinesterase inhibitor that facilitates neural transmission at the ganglionic level of both sympathetic and parasympathetic nerves. The drug is particularly useful in postviral POTS patients as well as patients with POTS secondary to a primary autoimmune disorder (such as Sjogren's syndrome or Lupus).

In severely affected patients, where other forms of therapy have either been poorly tolerated or ineffectual, we employ the drug erythropoietin. Originally developed for the treatment of anemia, it is also a potent vasoconstrictor that is quite useful in the treatment of orthostatic disorders. Due to the fact that erythropoietin (EPO) has to be administered by subcutaneous injection and because of its considerable expense, we normally reserve its use to patients who have proven refractory to or intolerant of other forms of therapy.

Another potential therapy is the somatostatin analog octreotide, which has potent vasoconstrictive effects. While some PD POTS patients may benefit from small doses of beta blockers (i.e., metoprolol 25 mg orally once or twice daily), the majority tend to feel worse on these agents.

The hyperadrenergic form of POTS is best treated by agents that block the release of norepinephrine or block its effects, such as clonidine. Labetalol is often effective, due to its alpha and beta blocking effects. Methyldopa is also helpful in select patients. Both the SSRIs and the norepinephrine reuptake inhibitors have been helpful in controlling patients' symptoms.

There is only limited data available at present on the prognosis of POTS patients. We are still in the process of following patients and analyzing their outcomes. Nonetheless some general trends have been noted. In patients suffering from postviral onset POTS, roughly one-half will make a good practical recovery over a 2–5 year period. Here, we define recovery as the relative absence of orthostatic symptoms with the ability to perform the normal activities of daily living with little or no restriction. However there are some patients that will not enjoy this degree of recovery and occasionally patients experience a progressive decline in functional status over time. In general, the younger the patient, the better the prognosis. In adolescents with the "developmental" form of POTS, approximately 75% will experience a significant recovery by the time they are in their early to mid-twenties. The vast majority of patients (~90%) will respond to a combination of physical therapy and pharmacotherapy. Patients with the hyperadrenergic form of POTS usually require treatment indefinitely. The prognosis of patients with the secondary POTS syndromes is usually determined by the prognosis of the underlying causative disorder.

The NDRF Handbook states that “Often these treatments, while helpful, do not bring the patients back to a sense of normal health.Over the course of months or even years, the patients can improve, or else they learn to cope with a chronic, debilitating, but not life-threatening disorder”.This extensive guide to the causes, symptoms, management, and coping for dysautonomias can be found at http://www.ndrf.org/NDRFHandbook.htm

So…now if you’ve gotten this far without falling asleep, you know some of the basics about dysautonomia and POTS.I could fill 200 pages with info about different ideas of what might be going on in POTS.More research is essential to getting anywhere.I’m going to stop now before everyone refuses to ever check my blog again!I hope this has been helpful in understanding a little more about what goes on in POTS and dysautonomia.

32 comments:

Lauren~Thanks for taking the time to post this much info about POTS. You and Adam popped into my head the other day for some reason and I was wondering how you were doing. I'm glad you finally have a name for the craziness you've been living with for the past few years. You are in my prayers. Keep it up!

Lauren, You are one tough chick even if you aren't from Ohio (ask adam). I am really glad that you created this blog to share your story and that you are feeling a little better. PS I keep "Chloe and the Rubber Band" on my favorites for whenever I need a laugh.

Thanks for your blog. I also suffer from POTS (post injury and surgery)and have read all the articles I could find (heck, I self diagnosed and then went to and Electrophysiology Cardiologist for a tilt table test to confirm the diagnosis). It took 6 different doctors to come up with a decent treatment plan and again, I had to do the research. At last I found a doc that works with me and so far I have not had as many bad episodes as I was having even a couple of months ago.

Keep up the good work in helping people with POTS and those that don't understand POTS.

Hi Nice Blog The human circulatory system is termed the cardiovascular system, from the Greek word kardia, meaning heart, and the Latin vasculum, meaningsmall vessel. The basic components of the cardiovascular system information are the heart, the blood vessels, and the blood. The work done by the cardiovascular system is astounding.

Hello. Your blog is totally freaking me out right now. I am a young practicing veterinarian in North Carolina. My husband's name is Adam. I have had symptoms of dysautonomia for many years. The symptoms were worst during puberty and became much better as I have aged. Recently, my headaches have started to increase in frequency and intensity again. Is there a doctor you recommend in this area (Raleigh/Durham)?

Thanks for the info. I also have POTS, symptoms starting about 18 years ago after being exposed to chemicals from a new car. I have the hyperadrenergic form, pretty extreme, with heart rates close to 200 within seconds of upright tilt. My heart rates would also increase by 40 to 50 bpm just with any kind of movement such as turning my head or even talking. I have gastroparesis which was severe for about 5 years (on feeding tube for a few years), improved with Chinese herbs. I still have frequent nausea and trouble swallowing but no more feeding tube. I was also in a wheelchair for about 2 years and couldn't even make it from one room to another in my house. I was eventually put on clonidine which has improved my symptoms enough to where I can walk for longer periods of time. I am still disabled. I still have symptoms on a daily basis (lots of symptoms), and I am not able to drive because of the dizziness but at least my heart rate isn't constantly at 200. I also have osteoporosis, TMJ, MVP, scoliosis and suspect I could have some kind of connective tissue disorder but my rheumatologist doesn't think so. I did take a steroid shot (decadron and kenalog) for allergies one time and this almost completely cleared up my symptoms for a few weeks but the next time I took the same dose of the same medications I had terrible side effects. I check on the latest research a few times a year and hope that soon there will be even better treatments available. By the way, I have been to the Mayo Clinic and Vanderbilt.

Thank you for the great info! I am definitely on the wrong meds and my doctors are clueless. The worst part is waiting months to get in to see "specialists" who look at me with a blank look and tell me there's nothing they can do. xo

Hi thank you for your blog.I'm 32 a single mother of 3 boys. January of this year I. Finally had team of 5 Dr's get together for all my symptoms. The hope is I may out grow it. Today I wait for disability and hope that when the symptoms appear that management will let me have a bit of life. Looking back over my life and health problems. I can see wear I've had my entire life. For last 2 plus years has taken me out of work. So I want to out grow management symptoms.Live my life free from all that goes with it. Yes after 2 LP multiple blood test. ms.3 MRI's. What a path. Your blog made me feel not crazy about living with dysautonomia. I really miss working. This week alone my HR at 97 plus. Spiking to 180 and BP was 73/59. Those are good for me. This has been sleep week and constipated sweating, and more last week was the total different end Now tonight is the first of many with no sleep. The joy's of my body not knowing. What it wants

I have found tools for body temperature. Flip flops and long sleeves. Then long socks for cold feet. And most of all the best of the best for me has been only last month ONB (nerve block). I just went to my neurologist and talk symptoms for now they work. Steroids spearing. And just started pionol. So have to do anuther EDG. What next a brake from Dr's. It took a total of 3 cardiac Dr's, 4 neurologist, 3 rumatoligest, endroconlogist, 5 Interests and 1 family. PCP. The best ENT, anthesgolist that took the time at 2 sinus surgery to say have your automatic nervous system. Looked at. SVT ablation I CAN GO ON ON. BEST NEWS I CAN OUT GROW IT!!!!!! SO IM TELLING MY SELF IVE OUT GROWN IT........

Sorry for misspelling. And typos. It's. 3am.Thank you again. I'm. Not crazy.

Great job with your blog!! THANKS!I hope you are getting better! With your attitude (positive), I think you will!!!I have dysautonomia as well and have learned lots of small secrets to avoid symptoms or to lessen or shorten them. I hope I can Share them soon with all of you! By the way I am a Doctor. Now I am having a relapse but as always.. I am working hard on getting out of it with:

1)patience

2) aerobic exercise (walking)(tailored to my individual capacity, functionality and exercise intolerance)I began with 10 min every other day and now do 25 min every other day, avoiding post exertional fatigue as much as possible not overtrainig myself.

3)No sweets. I try to eat only complex carbohydrates (those which take longer to be broken down into small sugar pieces by your body). Hole grain bread,pasta, All-Bran, etc.

4)Not starving, No fasting. It is beter to do frequent meals in moderate amounts. In vaerage each 4 hours (except while sleeping)

5)Limiting alcohol intake.

6)Not lying flat 100% (always have my head at least 15 cm higher than the rest of my body).At night it helps to sleep better.

7)Shower time in the morning: Temperaure not to hot (I know,... I would like it warmer too...).I always take a shower in a sitting position. I have a plastic cube (30 cm high) where I seat above. I keep my knees flexed. All this is to prevent venous blood pooling in my legs. We need the blood in our thoraxes and brains!Before shutting off the shower I take a 40 second cold shower while remaining seated (cold helps pheripheral vasoconstriction).I let the cold water hit my legs. During the cold shower I keep flexing my feet up and down (at the ankle point),helping the blood to return to my thorax and brain before standing up. I can also help it with massages, only ascending type. But muscle movement will work better as venous blood return depends primarily on the muscle pump generated by muscle contraction.

8)Brekfast: althogh I would love to have a warm drink I accepted that a cold drink doesn´t kill me as long as a hot drink. I mean,I I take a moderate amount of meal and a cold drink I do not feel like desperaten sleeping(lying flat or dying as much as when when I take a big big breakfast with a big cup of a hot drink.

9)I do not rush myself after eating, specially after breakfast.(I do not feel my best after eating). A good idea is sitting in a chair with my legs extended on a table/chair/sofa (at the level of my hips). Now that my blood has been gone to the digestive system, at least I help my body to recover that blood pooled in the leg veins. Not rushing to exercise, to house work or to standing after eating for 15-20 min helps me to cope with postprandial (post-eating) symptoms.

Ok, I have to go to a meeting. Hope to hear from all of you soon.Sara.

Just wanted to share my news! I recovered from POTS after going on a gluten free diet! To learn more about my recovery you can see my blog at www.dontmisdiagnoseme.blogspot.com. But if it worked for me, then it can work for others too! I hope by sharing this some of you may find your solution too. Please feel free to email me if you would like to at annie@nakedtruth.ca. I feel compelled to share my story so others might recover their health like me. It's like being born again. xoxo

Hi Trina, Happy by your great news!! I was testet for gluten intolerance but it was not my case. Anyway, because I just studied the subject recently (CELIAC DISEASE), please keep en eye (with your doctor advice of course)on Vitamin D levels. Its intestinal absortion may be impaired when celiac disease is active. Low vitamin D levels may cause severe symptoms as fatigue, muscle weakness, depression, bone pain, etc. Vitamin D is usually checked as 25 Hydroxi vitaminD (25(OH) Vitamin D).Good luck and enjoy health again!!! CONGRATULATIONS for being brave and having kept an eye on looking for answers!

Thank you for responding! I just wanted to mention that if you got a celiac test, it may have been a false negative. Only 5% of gluten intolerant people test positive for gluten intolerance. I had to get my kids tests through enterolab.com because their celiac tests came back negative but I wanted to be sure. And sure enough they are both gluten intolerant!

I was never tested for celiac, or if I was, it must have come back negative because I was never called in by a doctor to address it. I found out by going off gluten (and other common allergens - elimination diet). I noticed an improvement within 4 days. First my nausea lessened and my bloating went away. Then my dry eyes started to clear up and my energy levels started to increase. Within a week I was standing around the house just because I could! I would say I was about 80% better within a couple of weeks.

Sara, It's great to see your ideas for coping. Some really good ones in there and many that I depend on myself! Although I can't say that I can do that much exercise, but I'm trying to work my way up. Also, are you still able to practice as a doctor? If so, I am jealous! I'd love to hear more about your experience. Feel free to email me anytime at lmbutare@yahoo.com.

Trina,I'm so glad that you've had so much of an improvement on a gluten free diet! I was tested and was borderline, so I suspect I may have intolerance but not an allergy. I tried a GF diet for a month or 2 and unfortunately didn't see an improvement. I try to limit the amount I get each day, but it's a challenge when you feel awful. I'm so happy for you and your recovery!!

And I totally agree about Vitamin D3. I suspect that almost all of us have a deficiency of some degree (I know that I do) so it's definitely something to get checked by your doc.

Things I can think of as triggeners:1)Three consecutive days of 30 min walking without over exercicing or feeling tired during walking. Then did 4 sets of 15 sit ups and 4 sets of push ups(8 repetitions each time).Enough recovery time was allowd between each set of sit ups or push ups.No post exertional fatigue was felt after sport the first 3 days, but fatigue and dysautonmia symptoms flared up at the fourth day.(no exercise at that day because of fright of becoming worse and because of fatigue.

2) ALong the 3 previous days before symptoms and fatigue flare up I ate: gluten (pasta, bread, 5 beer (non alcohol beer), sugarless chocolate (3 pieces), half a glass of red wine, swiss cheese fondue, mexican quesadillas (ham,cheese). No other remarcable things that I can remember. Does any of these sound suspicious for causing symptom flare-up?

What may I have done wrong to become sick again? Opinions fully welcome!!!!....

Sleep was normal and mood was very good and relaxed. No anxiety felt during those days or on the relapse day.

Please feel free to comment about this mail and possible relapse triggers.

I got my diagnosis at the Mayo clinic in MN. I couldn't find anyone to help me or had any idea what they were doing until I went there. It was a lot of expense and time (2 weeks of testing for me), but worth it in the long run b/c I finally knew what I had. Hope this helps.

Lauren, Is it possible to contact you off this blog? I would have many questions to ask..., of course if you have energy to answer them. I am at the beginning of my dysautonomia diagnosis' journey. Your blog is very helpful - I appreciate the time and energy you invested into it.Thanks -Ursula

Hi, My name is Ashley and I'm 18 and my doctor thinks I have pots. I'm terrified, my mom is scared and i also have ehlers-danlos, would it be possible for me to contact you through your blog or a different way?

You are right about being gluten free. One responder to you indicated exactly why gluten free didn't work for her when she stated she tried to limit it as much as possible. It doesn't work that way, it must be 100%. don't rely of tests for gluten. They are not 100%. I tested negative too! With me gluten has a 2 day to 2 month delay in onset of different symptoms. 2 days for neurological, 4 days gastrointestinal, etc. Cure yourself by removing gluten and follow the Specific Carbohydrate Diet or Paleo, look it up. Ignore your doctors. I have my life back, so can you.

I agree about gluten. I don't have celiac disease, but I feel a thousand times better when I remove gluten. And you're right, you have to do it 100% for at least 10-14 days to notice any difference. I'm glad to hear that it has helped you too!

I'm so sorry that you are having symptoms that may be related to dysautonomia. I believe that you emailed me as well, so I will reply to your email instead of posting more here so that I can respond more in depth.

Why am I doing this?

I have been severely ill for 8 years. I was an active, healthy 26 year old emergency veterinarian loving life and my job. Everything crashed to a halt after a cat bite landed me in the hospital for a few days on IV antibiotics. Severe dizziness, fatigue, pain, and fainting were soon to follow. I had to take medical leave 3 months after I first became ill and was never able to work again.

It took more than 30 doctors, 2 years, and a trip to the Mayo clinic in Minnesota to finally get diagnosed with Postural Orthostatic Tachycardia Syndrome, or "POTS", a type of dysautonomia. I am still severely disabled, but fighting tooth and nail to get better every day. I want this blog to be a resource for others who have chronic "mystery" diagnoses or other chronic disorders that need help organizing their life again.

Bear with me on this blog because I am ill and will not be able to post as regularly as a "normal" person can!